The ASTRO-H X-ray Observatory

The joint JAXA/NASA ASTRO-H mission is the sixth in a series of highly successful X-ray missions initiated by the Institute of Space and Astronautical Science (ISAS). ASTRO-H will investigate the physics of the high-energy universe via a suite of four instruments, covering a very wide energy range, from 0.3 keV to 600 keV. These instruments include a high-resolution, high-throughput spectrometer sensitive over 0.3-2 keV with high spectral resolution of Delta E < 7 eV, enabled by a micro-calorimeter array located in the focal plane of thin-foil X-ray optics; hard X-ray imaging spectrometers covering 5-80 keV, located in the focal plane of multilayer-coated, focusing hard X-ray mirrors; a wide-field imaging spectrometer sensitive over 0.4-12 keV, with an X-ray CCD camera in the focal plane of a soft X-ray telescope; and a non-focusing Compton-camera type soft gamma-ray detector, sensitive in the 40-600 keV band. The simultaneous broad bandpass, coupled with high spectral resolution, will enable the pursuit of a wide variety of important science themes.Comment: 22 pages, 17 figures, Proceedings of the SPIE Astronomical Instrumentation "Space Telescopes and Instrumentation 2012: Ultraviolet to Gamma Ray

Emerging roles of ARHGAP33 in intracellular trafficking of TrkB and pathophysiology of neuropsychiatric disorders

Intracellular trafficking of receptor proteins is essential for neurons to detect various extracellular factors during the formation and refinement of neural circuits. However, the precise mechanisms underlying the trafficking of neurotrophin receptors to synapses remain elusive. Here, we demonstrate that a brain-enriched sorting nexin, ARHGAP33, is a new type of regulator for the intracellular trafficking of TrkB, a high-affinity receptor for brain-derived neurotrophic factor. ARHGAP33 knockout (KO) mice exhibit reduced expression of synaptic TrkB, impaired spine development and neuropsychiatric disorder-related behavioural abnormalities. These deficits are rescued by specific pharmacological enhancement of TrkB signalling in ARHGAP33 KO mice. Mechanistically, ARHGAP33 interacts with SORT1 to cooperatively regulate TrkB trafficking. Human ARHGAP33 is associated with brain phenotypes and reduced SORT1 expression is found in patients with schizophrenia. We propose that ARHGAP33/SORT1-mediated TrkB trafficking is essential for synapse development and that the dysfunction of this mechanism may be a new molecular pathology of neuropsychiatric disorders

Hitomi (ASTRO-H) X-ray Astronomy Satellite

The Hitomi (ASTRO-H) mission is the sixth Japanese x-ray astronomy satellite developed by a large international collaboration, including Japan, USA, Canada, and Europe. The mission aimed to provide the highest energy resolution ever achieved at E  >  2  keV, using a microcalorimeter instrument, and to cover a wide energy range spanning four decades in energy from soft x-rays to gamma rays. After a successful launch on February 17, 2016, the spacecraft lost its function on March 26, 2016, but the commissioning phase for about a month provided valuable information on the onboard instruments and the spacecraft system, including astrophysical results obtained from first light observations. The paper describes the Hitomi (ASTRO-H) mission, its capabilities, the initial operation, and the instruments/spacecraft performances confirmed during the commissioning operations for about a month

Factors that influence functional outcome after total or subtotal scapulectomy: Japanese Musculoskeletal Oncology Group (JMOG) study.

Scapulectomy requires not only joint resection but also wide resection of the shoulder girdle muscles. Even the significance of reconstruction has not yet been determined because of the difficulties in comparing the different conditions. The purpose of this study was to investigate factors that influence functional outcomes after scapulectomy in a multicenter study.This retrospective study comprised 48 patients who underwent total or subtotal scapulectomy and were followed for at least one year after surgery. Patients were registered at the Japanese Musculoskeletal Oncology Group affiliated hospitals. Soft tissue reconstruction for joint stabilization was performed when there was enough remaining tissue for reconstruction of the rotator cuff and tendons. In 23 cases, humeral suspension was performed. The average follow-up period was 61.9 months. Multivariate analysis was performed using the patient's background to determine which factors influence the Enneking functional score or active range of motion.The average functional score was 21.1 out of 30. Active shoulder range of motion was 42.7 degree in flexion, 39.7 degree in abduction, 49.6 degree of internal rotation and 16.8 degree of external rotation. The amount of remaining bone influenced functional outcome, which means that preserving the glenoid or the acromion lead to better function compared to total scapulectomy (p<0.01). Factors that influenced each functional measure include the amount of remaining bone, soft tissue reconstruction, the length of the resected humerus and nerve resection (p<0.05).Although shoulder function was almost eliminated following total or subtotal scapulectomy, minimal resection of bone, and soft tissue reconstruction should lead to better function

MM2 cortical form of sporadic Creutzfeldt-Jakob disease without progressive dementia and akinetic mutism : A case deviating from current diagnostic criteria

Sporadic Creutzfeldt-Jakob disease (sCJD) is classified into six types based on codon 129 polymorphism in the PRNP gene and the protease-resistant prion-related protein, PrP [1,2]. This classification corresponds well with the clinical course and the pathological findings. MM2-cortical type sCJD (MM2C-sCJD) is clinically characterized by slow progressive dementia, increased levels of 14-3-3 protein in the cerebrospinal fluid (CSF), and no periodic synchronous discharge (PSD) in electroencephalography [3]. We report the case of a patient presented with chronic progressive cortical symptoms. Based on the initial clinical findings, corticobasal syndrome (CBS) was suspected although he did not develop akinetic mutism during the lifetime. The postmortem pathological and anatomical findings confirmed MM2C-sCJD. As per the existing diagnostic criteria, our case was difficult to diagnose during the patient’s lifetime. Therefore, this is an important case for considering future revisions of the diagnostic criteria for MM2C-sCJD

Statistical analysis of all cases for Enneking functional score.

<p>Multivariate analysis was performed using seventeen factors of the patient’s background to determine which influence Enneking’s functional score or active range of motion for all cases and for total scapulectomy cases, separately. The amount of remaining bone influenced the Enneking functional score, which means that preserving the glenoid or the acromion lead to better function compared to total scapulectomy. However, there was no significant evidence that reconstruction improved total functional outcome.</p

Comparison of soft tissue reconstruction or no reconstruction after total scapulectomy.

<p>As for total scapulectomy cases, soft tissue reconstruction does not improve total functional score but does improve dexterity of the affected hand. Previously there was no clear evidence that soft tissue reconstruction after scapulectomy improved functional outcome.</p

New classification of scapulectomy.

<p>Five categories are created in terms of resection area. Preserving glenoid or acromion lead to better function compared to total scapulectomy. Preoperative planning with this classification will contribute to expected postoperative function.</p

Summary of multivariate analysis.

<p>Factors that influenced functional outcome include the amount of remaining bone, soft tissue reconstruction, length of the resected humerus and nerve resection. As for total scapulectomy cases, soft tissue reconstruction did not lead to better total functional score but did allow better dexterity of affected hand. This result encourages doing soft tissue reconstruction after scapulectomy, which had been uncertain to improve functional outcome.</p

Patient’s characteristics and functional outcomes.

<p>This retrospective study comprised 48 patients who underwent total or subtotal scapulectomy (more than half of the scapula was resected) and followed for at least one year after surgery. Patients were registered at the Japanese Musculoskeletal Oncology Group affiliated hospitals. Using the Enneking functional score, function and hand position, which reflect shoulder ability, had low scores, but pain and dexterity, which reflect usefulness of the hand joints, had satisfactory scores. The mean total score was 21.1 out of 30 (12–30), which overall is a satisfactory score following resection of the shoulder girdle. Data are expressed as mean±SD.</p